Dieulafoy's disease of the bronchial tree: a case report / Doença de Dieulafoy da árvore brônquica: relato de caso

ABSTRACT CONTEXT: Dieulafoy's disease of the bronchial tree is a very rare condition. Few cases have been reported in the literature. It can be asymptomatic or manifest with massive hemoptysis. This disease should be considered among heavy smokers when recurrent massive hemoptysis is present amid otherwise normal findings. The treatment can be arterial embolization or surgical intervention. CASE REPORT: A 16-year-old girl was admitted to the emergency department due to hemoptysis with an unknown lesion in the bronchi. She had suffered massive hemoptysis and respiratory failure one week before admission. Fiberoptic bronchoscopy revealed a lesion in the bronchus of the right lower lobe, which was suspected to be a Dieulafoy lesion. Segmentectomy of the right lower lobe and excision of the lesion was carried out. The outcome for this patient was excellent. CONCLUSION: Dieulafoy's disease is a rare vascular anomaly and it is extremely rare in the bronchial tree. In bronchial Dieulafoy's disease, selective embolization has been suggested as a method for cessation of bleeding. Nevertheless, standard anatomical lung resection is a safe and curative alternative.

RESUMO CONTEXTO: A doença de Dieulafoy da árvore brônquica é uma condição muito rara, poucos casos foram descritos na literatura. Pode ser assintomática ou manifestar-se com hemoptise maciça. Esta doença deve ser considerada em fumadores pesados quando eles têm recorrentes hemoptises maciças sem outros achados anormais. O tratamento pode ser tanto embolização arterial como intervenção cirúrgica. RELATO DE CASO: Uma menina de 16 anos foi admitida no Serviço de Urgências devido a hemoptise com uma lesão nos brônquios de origem desconhecida. Havia sofrido hemoptise maciça e insuficiência respiratória uma semana antes da admissão. A broncoscopia de fibra óptica relevou lesão no brônquio do lobo inferior direito, com suspeita de ser lesão de Dieulafoy. Foi realizada uma segmentectomia do lobo inferior direito com excisão da lesão. O resultado da paciente foi excelente. CONCLUSÃO: A doença de Dieulafoy é uma anomalia vascular rara, sendo extremamente rara na árvore brônquica. Na doença de Dieulafoy bronquial, embolização seletiva tem sido sugerida como método para cessação do sangramento; no entanto, a habitual resseção anatômica do pulmão é uma alternativa segura e curativa.

Dieulafoy's disease of the bronchial tree: a case report.

CONTEXT:: Dieulafoy's disease of the bronchial tree is a very rare condition. Few cases have been reported in the literature. It can be asymptomatic or manifest with massive hemoptysis. This disease should be considered among heavy smokers when recurrent massive hemoptysis is present amid otherwise normal findings. The treatment can be arterial embolization or surgical intervention. CASE REPORT:: A 16-year-old girl was admitted to the emergency department due to hemoptysis with an unknown lesion in the bronchi. She had suffered massive hemoptysis and respiratory failure one week before admission. Fiberoptic bronchoscopy revealed a lesion in the bronchus of the right lower lobe, which was suspected to be a Dieulafoy lesion. Segmentectomy of the right lower lobe and excision of the lesion was carried out. The outcome for this patient was excellent. CONCLUSION:: Dieulafoy's disease is a rare vascular anomaly and it is extremely rare in the bronchial tree. In bronchial Dieulafoy's disease, selective embolization has been suggested as a method for cessation of bleeding. Nevertheless, standard anatomical lung resection is a safe and curative alternative.

Tracheobronchopathia osteochondroplastica.

Tracheobronchopathia osteochondroplastica is a rare benign disease, of unknown cause, characterized by numerous sessile, cartilaginous, or bony submucosal nodules distributed throughout the anterolateral walls, projecting into the laryngotracheobronchial lumen. In general, tracheobronchopathia osteochondroplastica is diagnosed incidentally during bronchoscopy or autopsy and is not associated with a specific disease. We report the case of a male patient who was diagnosed with tracheobronchopathia osteochondroplastica via bronchoscopy and biopsy. RESUMO A traqueobroncopatia osteocondroplástica é uma doença benigna rara, de causa desconhecida, caracterizada por numerosos nódulos submucosos sésseis, cartilaginosos e/ou ósseos, distribuídos pelas paredes anterolaterais da traqueia, projetando-se no lúmen laringotraqueobrônquico. Em geral, a traqueobroncopatia osteocondroplástica é descoberta acidentalmente durante broncoscopias ou em necropsias e não é associada a uma doença específica. Relatamos o caso de um paciente que foi diagnosticado com traqueobroncopatia osteocondroplástica por broncoscopia e biópsia.

Tracheobronchopathia osteochondroplastica / Traqueobroncopatia osteocondroplástica

ABSTRACT Tracheobronchopathia osteochondroplastica is a rare benign disease, of unknown cause, characterized by numerous sessile, cartilaginous, or bony submucosal nodules distributed throughout the anterolateral walls, projecting into the laryngotracheobronchial lumen. In general, tracheobronchopathia osteochondroplastica is diagnosed incidentally during bronchoscopy or autopsy and is not associated with a specific disease. We report the case of a male patient who was diagnosed with tracheobronchopathia osteochondroplastica via bronchoscopy and biopsy.

RESUMO A traqueobroncopatia osteocondroplástica é uma doença benigna rara, de causa desconhecida, caracterizada por numerosos nódulos submucosos sésseis, cartilaginosos e/ou ósseos, distribuídos pelas paredes anterolaterais da traqueia, projetando-se no lúmen laringotraqueobrônquico. Em geral, a traqueobroncopatia osteocondroplástica é descoberta acidentalmente durante broncoscopias ou em necropsias e não é associada a uma doença específica. Relatamos o caso de um paciente que foi diagnosticado com traqueobroncopatia osteocondroplástica por broncoscopia e biópsia.

Tomographic and functional findings in severe COPD: comparison between the wood smoke-related and smoking-related disease.

OBJECTIVE: Wood smoke exposure is a risk factor for COPD. For a given degree of airway obstruction, the reduction in DLCO is smaller in individuals with wood smoke-related COPD than in those with smoking-related COPD, suggesting that there is less emphysema in the former. The objective of this study was to compare HRCT findings between women with wood smoke-related COPD and women with smoking-related COPD. METHODS: Twenty-two women with severe COPD (FEV1/FVC ratio < 70% and FEV1 < 50%) were divided into two groups: those with wood smoke-related COPD (n = 12) and those with smoking-related COPD (n = 10). The two groups were compared regarding emphysema scores and airway involvement (as determined by HRCT); and functional abnormalities-spirometry results, DLCO, alveolar volume (VA), the DLCO/VA ratio, lung volumes, and specific airway resistance (sRaw). RESULTS: There were no significant differences between the two groups in terms of FEV1, sRaw, or lung hyperinflation. Decreases in DLCO and in the DLCO/VA ratio were greater in the smoking-related COPD group subjects, who also had higher emphysema scores, in comparison with the wood smoke-related COPD group subjects. In the wood smoke-related COPD group, HRCT scans showed no significant emphysema, the main findings being peribronchial thickening, bronchial dilation, and subsegmental atelectasis. CONCLUSIONS: Female patients with severe wood smoke-related COPD do not appear to develop emphysema, although they do show severe airway involvement. The reduction in DLCO and VA, with a normal DLCO/VA ratio, is probably due to severe bronchial obstruction and incomplete mixing of inspired gas during the determination of single-breath DLCO.

Novel silicone stent to treat tracheobronchial lesions: results of 35 patients.

We describe a case series of 35 patients with either benign (14) or malignant (21) tracheal stenosis who were treated using a novel silicone stent, the HCPA-1, designed to prevent migration. Between March 2001 and September 2008, 13 women and 22 men received 41 HCPA-1 stents. The median duration of stenting in benign cases was 457 days (range, 4-2,961 days). Successful stent removal with curative results was accomplished in 2 patients with tracheomalacia and 1 with post-intubation stenosis. In malignant cases, the median duration of stenting was 162 days (range, 1-1,279 days). Five patients had tumor progression with obstruction requiring repeated laser resection, dilatation, or additional stents. Two patients died due to airway obstruction despite bronchoscopic intervention. Twelve patients with malignant lesions died with the stent in place. At the end of the study, 3 patients with malignant disease remained alive; 2 were lost to follow-up. The HCPA-1 stent proved to be safe, with no severe complications during the study period, and effective in improving quality of life with relief of dyspnea.

[Diffuse abnormalities of the trachea: computed tomography findings]. / Alterações difusas da traquéia: aspectos na tomografia computadorizada.

The aim of this pictorial essay was to present the main computed tomography findings seen in diffuse diseases of the trachea. The diseases studied included amyloidosis, tracheobronchopathia osteochondroplastica, tracheobronchomegaly, laryngotracheobronchial papillomatosis, lymphoma, neurofibromatosis, relapsing polychondritis, Wegener's granulomatosis, tuberculosis, paracoccidioidomycosis, and tracheobronchomalacia. The most common computed tomography finding was thickening of the walls of the trachea, with or without nodules, parietal calcifications, or involvement of the posterior wall. Although computed tomography allows the detection and characterization of diseases of the central airways, and the correlation with clinical data reduces the diagnostic possibilities, bronchoscopy with biopsy remains the most useful procedure for the diagnosis of diffuse lesions of the trachea.

Dynamic CT evaluation of the central airways in patients undergoing tracheoplasty for tracheobronchomalacia.

OBJECTIVE: The objective of this study was to describe the role of pre- and postoperative dynamic CT in patients undergoing tracheoplasty, a novel surgical method for treatment of severely symptomatic tracheobronchomalacia. CONCLUSION: Five patients were referred for dynamic MDCT before and after undergoing tracheoplasty at our institution. Preoperatively, all patients showed signs of tracheobronchomalacia (> or = 50% airway collapse) on bronchoscopy, and four (80%) of these five patients showed evidence of malacia on dynamic forceful expiratory CT scans. In all five cases, postoperative CT showed a reduction in the degree of airway collapse during expiration, changes in shape of the trachea during inspiration, and posterior wall thickening related to the procedure. Our preliminary results suggest a potentially important role for CT in the pre- and postoperative assessments of patients with tracheobronchomalacia referred for tracheoplasty.